Brugada syndrome

Pathology

Brugada syndrome is a form of inherited cardiovascular disease with may present with sudden cardiac death. It is inherited in an autosomal dominant fashion and has an estimated prevalence of 1:5,000-10,000. Brugada syndrome is more common in Asians.

Pathophysiology

a large number of variants exist
around 20-40% of cases are caused by a mutation in the SCN5A gene which encodes the myocardial sodium ion channel protein

ECG changes

convex ST segment elevation > 2mm in > 1 of V1-V3 followed by a negative T wave
partial right bundle branch block
changes may be more apparent following flecainide


© Image used on license from Dr Smith, University of Minnesota

ECG showing Brugada pattern, most marked in V1, which has an incomplete RBBB, a downsloping ST segment and an inverted T wave

Management

implantable cardioverter-defibrillator